How would you approach the treatment of a patient who, during a hospitalization, was initially diagnosed with TTP and treated with PLEX with good response, but renal biopsy then resulted class IV LN along with changes of thrombotic microangiopathy?
Answer from: at Academic Institution
This is a challenging scenario; the literature consists of case reports and case series. In clinical practice, combining PLEX with cyclophosphamide or Rituximab is a possibility.I was recently involved in a case of an SLE patient with refractory TTP (no nephritis) where Caplacizumab (Scully et al.,P...
I agree with my colleague, Dr. @Broder’s response and provide the following comments. A diagnosis of TTP suggests this patient had ADAMTS 13 of less than 5% and based on the kidney biopsy this likely occurred in the setting of SLE with secondary TTP (i.e., acquired immunoglobulin inhibitor of ...
We had a similar patient who eventually turned out to have just lupus, not TTP as initially diagnosed. The ADAMTS levels when back did not support TTP, and the antiphospholipid antibodies were also positive in addition to other autoimmune serologies. She had biopsy-proven lupus nephritis, and presen...